SOHO State of the Art Updates and Next Questions | Challenging Scenarios in the Management of Myeloproliferative Neoplasms.
Philadelphia-chromosome negative (Ph-neg) myeloproliferative neoplasms (MPNs) carry a variable rate of progression to the fibrotic stage of disease and/or the accelerated/blast-phase (AP/BP) of disease. Many of the challenging management scenarios that arise in the treatment of MPNs occur in those with higher-risk myelofibrosis (MF) or MPN-AP/BP. In this review we will focus upon 3 challenging clinical scenarios pertinent to the management of high-risk MPNs. We discuss how to incorporate molecular data into decision making around allogeneic hematopoietic stem cell transplantation in MF, strategies to address splenomegaly in patients with MF with inadequate response to an initial JAK inhibitor, and what sort of treatment approaches can be employed in the management of MPN-AP/BP.